Long QT syndrome is associated with lethal tachyarrhythmia that can lead to syncope, seizure, and sudden death. Congenital long QT syndrome is a genetic disorder, characterized by delayed cardiac repolarization and prolongation of the QT interval on the electrocardiogram (ECG). Type 2 congenital long QT is linked to mutations in the human ether a go-go-related gene (HERG). There are environmental triggers of adverse cardiac events such as emotional and acoustic stimuli, but fever can also be a potential trigger of life-threatening arrhythmias in long QT syndrome type 2 patients. Herein, we report a healthy young man who experienced fever-induced polymorphic ventricular tachycardia and QT interval prolongation.
Adult ; Electrocardiography ; Fever/*complications ; Humans ; Long QT Syndrome/*diagnosis/*etiology ; Male ; Ventricular Fibrillation/*diagnosis/*etiology

PURPOSE: In some patients with an implantable cardioverter defibrillator (ICD), multiple episodes of electrical storm (ES) can occur. We assessed the prevalence, features, and predictors of ES in patients with ICD. MATERIALS AND METHODS: Eighty-five patients with an ICD were analyzed. ES was defined as the occurrence of two or more ventricular tachyarrhythmias within 24 hours. RESULTS: Twenty-six patients experienced at least one ES episode, and 16 patients experienced two or more ES episodes. The first ES occurred 209 +/- 277 days after ICD implantation. In most ES cases, the index arrhythmia was ventricular tachycardia (65%). There were no obvious etiologic factors at the onset of most ES episodes (57%). More patients with a structurally normal heart (p = 0.043) or ventricular fibrillation (VF) as the index arrhythmia (p = 0.017) were in the ES-free group. Kaplan-Meier estimates and a log-rank test showed that patients with nonischemic dilated cardiomyopathy (DCMP) (log-rank test, p = 0.016) or with left ventricular ejection fraction < 35% (p = 0.032) were more likely to experience ES, and that patients with VF (p = 0.047) were less affected by ES. Cox proportional hazard regression analysis showed that nonischemic DCMP correlated with a greater probability of ES (hazard ratio, 3.71; 95% confidence interval, 1.16-11.85; p = 0.027). CONCLUSION: ES is a common and recurrent event in patients with an ICD. Nonischemic DCMP is an independent predictor of ES. Patients with VF or with a structurally normal heart are less likely to experience ES.
Adult ; Aged ; Defibrillators, Implantable/*adverse effects ; Female ; Humans ; Male ; Middle Aged ; Tachycardia, Ventricular/*diagnosis/etiology ; Ventricular Fibrillation/*diagnosis/etiology

A 53-year-old woman was referred for ventricular fibrillation with resuscitation. A CT-angiography showed signs of a right ventricular enlargement without obvious cause. A cardiac MRI demonstrated a dilated and hypokinetic right ventricle with extensive late gadolinium enhancement. Arrhythmogenic right ventricular dysplasia (ARVD) was suspected according to the "revised ARVD task force criteria". An endomyocardial biopsy was inconclusive. The patient developed purulent pericarditis after epicardial ablation therapy and died of toxic shock syndrome. The post-mortem pathologic examination demonstrated sarcoidosis involving the heart, lungs, and thyroid gland.
Arrhythmogenic Right Ventricular Dysplasia/*diagnosis ; Female ; Heart Ventricles/pathology ; Humans ; Lung/pathology ; Magnetic Resonance Imaging ; Middle Aged ; Myocardium/pathology ; Sarcoidosis/*diagnosis ; Thyroid Gland/pathology ; Ventricular Fibrillation/diagnosis/*etiology